Severely Altered-Consciousness Status and Profuse Vomiting in Infants Food Protein–Induced Enterocolitis Syndrome (FPIES) a Challenging Diagnosis Judith Barasche, MD,* Fabiola Stollar, MD,* Marcel M. Bergmann, MD,*† and Jean-Christoph Caubet, MD* Abstract: In infants, the causes of acute repetitive vomiting and severely altered-consciousness status include a broad differential diagnosis, that is, primarly sepsis, infectious gastroenteritis, head injury, and intoxication, as well as neurologic, metabolic, and cardiologic condition diseases. In pa- tients developing such symptoms, allergy as an etiological cause is often not considered by primary care physicians. With this case report, we aim to draw the attention of general pediatricians, emergency physicians, and intensivists to the fact that non–immunoglobulin E–mediated food allergic gastrointestinal disorders such as food protein–induced enterocolitis syn- drome should be considered in patients with sepsis-like symptoms. Key Words: food protein–induced enterocolitis syndrome, food allergy, children (Pediatr Emer Care 2016;00: 00–00) CASE In infants, the causes of acute repetitive vomiting and se- verely altered-consciousness status include a broad differential di- agnosis, that is, primarly sepsis, infectious gastroenteritis, head injury and intoxication, as well as neurologic, metabolic, and car- diologic condition diseases (Table 1). 1 In patients developing such symptoms, allergy as an etiological cause is often not considered by primary care physicians. We aim to draw the attention of gen- eral pediatricians, emergency physicians, and intensivists to the fact that non–immunoglobulin E (IgE)–mediated food allergic gastrointestinal disorders such as food protein–induced enteroco- litis syndrome (FPIES) should be considered in patients with sepsis-like symptoms. Because FPIES is a diagnosis of exclusion without confirma- tory testing in the acute setting, clinicians should have a better un- derstanding of the presentation and maintain a high level of suspicion to make this diagnosis. We present a case of an infant with a final diagnosis of FPIES, who underwent many invasive investigations and intensive care hospitalization. We aim to demonstrate the severity with which FPIES can present, the importance of a detailed history in reaching this diagnosis, and the negative consequences of delayed or missed diagnosis of FPIES. A healthy 8-month-old boy without allergic history presented to the emergency department with acute onset of profuse emesis and asthenia. Symptoms began abruptly 1 hour after ingestion of a meal with chicken and milk. Parents denied recent illness, toxic exposures, or head injury. Physical examination was characterized by pallor and lethargy with a Glasgow Coma Scale between 9 and 10. A very broad differential diagnosis was discussed by the emergency pediatricians and led to various investigations. The blood gas analysis highlighted a metabolic acidosis (ph 7.26; PCO 2 6.9 kPa; HCO 3 -22.2 mEq/L; Base Excess (BE) -3.9), and the complete blood count showed leukocytosis (26.8 G/L) with left shift of the polymorphonuclear leukocytes (ie, nonsegmented neutrophils of 1.07 G/L) and thrombocytosis (663 G/L). An ab- dominal x-ray and ultrasonography did not reveal any significant anomaly. Cerebrospinal fluid analysis, including white blood cell count and protein, and head computed tomography were normal. Our patient was rapidly treated with an intravenous bolus of normal saline solution (20 mL/kg) and was transferred to the in- tensive care unit because of fluctuating levels of consciousness. An hour after admission, the patient's clinical condition improved significantly, particularly his state of consciousness; thus, antibi- otics were not given at that time. After a few hours, the boy devel- oped abundant and odorous liquid stools. Occult blood testing was positive. At 48 hours, although the leukocytes were in the refer- ence range (9.8 G/L), a normocytic normochromic anemia (hemo- globin of 90 g/L) was detected. Of note, stool bacterial culture and viral tests were negative. After 5 days of extensive and unrevealing workup, food al- lergy was suspected on the basis of a more precise clinical history revealing a similar milder reaction after chicken ingestion, several weeks before. Indeed, our patient had 1 episode of intractable vomiting after ingestion of a meal containing chicken. He did not receive this food between those 2 episodes. An allergic workup including skin prick test and specific IgE to cow’s milk and chicken was negative. Because of a low index of suspicion (ie, no ingestion of milk during the first episode), a food challenge to cow’s milk was performed and was negative. On the basis of clinical diagnostic criteria, FPIES to chicken was diagnosed. 2 Food protein–induced enterocolitis syndrome is a relatively common severe non–IgE-mediated food allergy, with an esti- mated prevalence of 0.34% for milk-FPIES in a birth cohort from Israel. 3 Its diagnosis is based on clinical criteria defined by Powell in 1978 and recently revised by several groups. 2 However, this disorder is clearly underdiagnosed, partly due to the lack of knowledge of pediatricians, but also due to the lack of diagnostic tests. 4 Indeed, although elevated white cell with a left shift, thrombocytosis, and methemoglobinemia have been described in a subset of patients, these tests are not specific to diagnose From the *Department of Child and Adolescent, University Hospitals of Ge- neva and Medical School of The University of Geneva, Geneva, Switzerland; and †Centro Pediatrico del Mendrisiotto, Mendrisio, Switzerland. Disclosure: The authors declare no conflict of interest. All authors are responsible for the reported case report. Judith Barasche wrote the first draft of the article. All authors have contributed significantly to the concept, drafting, and revising of the article. All authors have read and accepted the article for submission. No honorarium, grant, or other form of payment was given to anyone to produce the present article. The authors declare having no conflicts of interest regarding this article. No sponsor has been implicated in this case report and at any stage (collection, analysis, and interpretation of data; writing of the manuscript; or decision to submit the article for publication). This article or any of its data have not been published previously. The present article is not under consideration elsewhere and will not be submitted elsewhere while under consideration by the Pediatric Emergency Care. Reprints: Jean-Christoph Caubet, MD, Pediatric Allergy Unit, Geneva University Hospital, 6 rue Willy-Donzé, CH-1211 Geneva14, Switzerland (e‐mail: Jean-Christoph.Caubet@hcuge.ch). Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0749-5161 ILLUSTRATIVE CASE Pediatric Emergency Care • Volume 00, Number 00, Month 2016 www.pec-online.com 1 Copyright © 2016 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.